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Objectives: Age-related macular degeneration (AMD) is a progressive disorder among people aged ≥50 years. Some dietary factors associated with the susceptibility to AMD include dietary glyc...

Context: Syringobulbia is a very rare progressive disorder of central nervous system, with several possible underlying conditions. Rarely, it is also encountered as a late complication of syringomyelia. Findings: In the present manuscript, a case of a paraplegic patient, due to traumatic spinal cord injury (thoracolumbar fracture), presenting after years progressively developing symptoms of the lower cranial nerves and upper extremities, owed to syringomyelia and syringobulbia, the surgical treatment applied and its outcomes are described. We performed a syringo-peritoneal shunting procedure using a T-tube. The patient's symptoms resolved postoperatively and the cavity's size was reduced to a great degree. Conclusion/Clinical Relevance: The late appearance of cranial nerve deficits or symptoms—signs of the upper extremities in a patient with traumatic thoracic spinal cord injury should raise suspicion that post-traumatic syringomyelia or syringobulbia has occurred. In such cases, radiologic evaluation and early surgical drainage of the cyst as a means of preventing significant delayed neurologic deficit is advocated.

Primary progressive aphasia (PPA) is a progressive disorder of language that is increasingly recognised as an important presentation of a specific spectrum of neurodegenerative conditions.In an era of etiologically specific treatments for neurodegenerative conditions, it is crucial to establish the histopathologic basis for PPA. In this review, I discuss biomarkers for identifying the pathology underlying PPA.Clinical syndromes suggest a probabilistic association between a specific PPA variant and an underlying pathology, but there are also many exceptions. A considerable body of work with biomarkers is now emerging as an important addition to clinical diagnosis. I review genetic, neuroimaging and biofluid studies that can help determine the pathologic basis for PPA.Together with careful clinical examination, there is great promise that supplemental biomarker assessments will lead to accurate diagnosis of the pathology associated with PPA during life and serve as the basis for clinical trials in this spectrum of disease.

Chronic obstructive pulmonary disease (COPD) is a progressive disorder that affects 300 million people worldwide and is responsible for 3 million deaths annually. Currently, there are no accepted biomarkers of COPD, which has impaired drug development and management of patients with COPD. Pneumoproteins, which are proteins synthesized predominantly in the lungs, are promising blood biomarkers because they have high specificity for lung disease. The most promising is surfactant protein-D, which is synthesized largely in Type 2 pneumocytes, and its blood concentrations have been associated with COPD and with certain clinical end points such as mortality. In this paper, we discuss surfactant protein-D and other pneumoproteins as promising biomarkers of COPD.

Amyotrophic lateral sclerosis (ALS), a progressive disorder that usually runs a fatal course within 2–5 years of onset, is characterized by loss of motor neurons in the cortex, brain stem, and spinal cord. In this study mercury (Hg) concentrations were determined in blood samples of 40 ALS patients, of whom 24 ALS patients were from the Ganga plain region and their metal levels were found to be significantly higher. In contrast, Hg levels in the 16 ALS patients from non-Ganga plain regions whose levels were similar to those of the control subjects. Mercury in stream water samples and freshly deposited stream sediment were determined in the locations of patients’ native residences along the Ganga plain region and metal levels were found to be higher than the threshold guideline values for Hg in stream waters recommended by WHO. The geo-accumulation index in selected sites of freshly deposited stream sediment ranged from moderate to highly polluted. The high levels of Hg observed in ALS patients may potenti...

Background: Previous studies looking at relearning and retention of word labels in people with semantic dementia have shown some improvement in naming immediately after the period of learning but this has not usually been maintained. Studies have also shown rigid learning of names, in the order of presentation and to the picture exemplars only, with no generalisation of learning. Aims: This study aimed to explore relearning of a small vocabulary set in a person with semantic dementia (CUB) and to examine her ability to generalise this learning. In addition, it aimed to find out how long the learning persisted after therapy was completed given that semantic dementia is a progressive disorder. Methods & Procedures: A single-case design was used where CUB was asked to learn 28 words while a further 28 were left as controls. A "look and say" method was used daily for 1 month. As well as examining learning of the therapy and control set, CUB was asked to name 168 other exemplars of the learning set to see whether there had been any transfer of her learning from the therapy set. Outcomes & Results: CUB not only relearned a set of picture names but retained these without deliberate practice over a 6-month period. She was also able to generalise this learning to other visually similar exemplars in testing and in daily use. The maintenance of relearning was achieved despite severe deterioration in her semantic memory. Conclusions: Possible reasons are explored as to why CUB was able to relearn and retain these words and why this may differ from all previously reported cases. Differences in amount of time spent relearning, number of items learned, therapy methods, the severity of semantic memory impairment, the degree of atrophy, and the behavioural profiles of people with semantic dementia do not provide adequate explanations for our individual's differential ability to retain her learning over 6 months. The most plausible explanation is that the person with semantic dementia generalised her learning to her everyday speech and this provided the source of maintenance for the relearned names.

Pathological gambling is classified in the Diagnostic and Statistical Manual of Mental Disorders, 4th Edition as an impulse-control disorder. In the International Classification of Diseases of the WHO, pathological gambling is coded under the heading of 'Habit and Impulse Disorders'. Pathological gambling is a chronic, progressive disorder, which has a prevalence of 1-3.4% among western civilizations. The enormous personal and social consequences of this disorder include a high rate of suicide attempts, job loss, marital and family problems, legal problems, and criminal behavior. Recent studies have demonstrated that pathological gambling patients respond well to treatment with selective serotonin reuptake inhibitors, mood stabilizers and opioid antagonists. These findings support the idea that pathological gambling and other disorders of impulse control may be conceptualized as part of the obsessive-compulsive spectrum disorders or addictive disorders. This article will discuss possible treatment strategies according to different behavior patterns in pathological gambling and also remind the physicians who intend to treat this disorder of the possible diagnosis of pathological gambling.

Schizophrenia is a chronic progressive disorder that has at its origin structural brain changes in both white and gray matter. It is likely that these changes begin prior to the onset of clinical symptoms in cortical regions, particularly those concerned with language processing. Later, they can be detected by progressive ventricular enlargement. Current magnetic resonance imaging (MRI) technology can provide a valuable tool for detecting early changes in cortical atrophy and anomalous language processing, which may be predictive of who will develop schizophrenia.

Migraine may be conceptualized, not just as an episodic disorder, but as a chronic recurrent and sometimes chronic progressive disorder. Transformed migraine, often referred to as chronic migraine is the result of migraine progression. This article will review the clinical features of transformed migraine, highlighting that its phenotype varies according to stage. Early in the process of transformation, attacks with migraine features are very common. As the disease evolves, most attacks lack the migraine features. The risk factors for migraine progression and the mechanisms for progression will be discussed. This review concludes with the prospects for treating transformed migraine and avoiding migraine progression.

Medial temporal lobe epilepsy due to hippocampal sclerosis is the most common epileptic syndrome and, if medically refractory, is a progressive disorder. Advances over the past decade allow this clinicopathological syndrome to be diagnosed in vivo. Many patients with hippocampal sclerosis become refractory to antiepileptic medications and are at risk of progressive hippocampal damage, cognitive deterioration and other disabling manifestations of refractory epilepsy. Fortunately, if hippocampal sclerosis is detected early and treated surgically, most patients with this syndrome can be rendered seizure-free, thus sparing them from further progression and disability.

Parkinson's disease is a progressive disorder that may ultimately lead to severe disability. Its course may be slowed or arrested by neuroprotective interventions that influence the basic mechanisms involved in neuronal death. In the last 2 years, claims have been made for several possible neuroprotective treatments, and patients and physicians are increasingly enquiring about such a therapy. The present report summarizes the basis of various potential neuroprotective strategies and the progress that has been made in establishing their validity and clinical utility.

Dementia is a chronic progressive disorder that necessitates an interdisciplinary team approach to provide the highest quality of health care. The purpose of this article is to describe and promote the collaboration of psychologists and occupational therapists as key interdisciplinary team members. Multiple sources were referenced to develop and describe an interdisciplinary team model. Occupational therapists possess skills in understanding function, maximizing residual strengths, defining small changes, modifying the environment, and developing caregiver strengths and compensations. These skills can complement the skills of psychologists in understanding behavior and emotion, developing behavioral interventions, supporting team development, and providing psychosocial support to caregivers.

Parkinson's Disease (PD) is a chronic, progressive disorder of the central nervous system characterized by tremors, stiffness, slowness of movement, and impaired balance and coordination. It affects over one million people in the U.S. today, including such personalities as actor Michael J. Fox, former boxer Muhammad Ali, and evangelist Billy Graham. At present, the cause and the cure for PD remain elusive but active, promising research is being done in this field. This article discusses selected sites and resources available on the Web for PD.

Alcoholism is a common, chronic, often progressive disorder that has negative effects on a patient's health and severe consequences for society as well. A positive, public health approach that integrates medical, psychological, and social therapies can lead to improved outcomes for patients who abuse alcohol. Physicians can play an important role by educating patients to prevent alcohol abuse from starting, being alert to the risk factors, recognizing the signs of alcoholism (especially during its early stages), and initiating interventions designed to halt progression of this disease. Doctors should maintain a therapeutic stance with patients who have continued to abuse alcohol, even after frequent relapses. Consultation with alcoholism experts may be helpful when treatment is difficult or there is the possibility of a dual diagnosis.

Jessie Khera,1 Nadine E Andrew,1 Dominique A Cadilhac,1,2 Tara Purvis,1 Michael C Fahey,3,4 Hyam Barry Rawicki1 1Department of Medicine, Faculty of Medicine, Nursing and Health Sciences, Monash University, Clayton, 2Stroke Division, Florey Institute of Neurosciences and Mental Health, Heidelberg, 3Department of Paediatrics, Faculty of Medicine, Nursing and Health Sciences, Monash University, Clayton, 4Department of Medicine, Melbourne University, Parkville, VIC, Australia Objective: Hereditary spastic paraplegia (HSP) is a rare progressive disorder with few treatment options. We aim to describe the effect of continuous intrathecal baclofen (ITB) pump therapy on the clinical and functional outcomes of patients with HSP. Methods: This is a retrospective study, using medical record audit data. Adult patients with HSP who had received ITB trial or therapy and had pre- and post-ITB assessment data available were eligible for inclusion. A purposefully designed audit tool was used. Patients with a successful trial received an ITB implantable SynchroMed® II pump. Demographic, clinical, and outcome data were obtained pre- and post-pump trial and pump insertion. Functional, spasticity, and mobility measures were compared pre- and post-ITB trial and pre- and post-ITB pump insertion. Results: Data for nine patients were available. Six were male and the median age was 55 years (Q1, Q3: 46, 55). All received an ITB trial, and those who responded favorably (n=8) had an ITB pump inserted. Following ITB therapy, improvements were demonstrated for rectus femoris (P=0.04) and gastrocnemius spasticity measures (P=0.03). All patients reported subjective improvements in function, and three of the four with pre- and post-pump assessments, demonstrated clinically meaningful improvements in mobility. Side effects were minimized with appropriate dose titrations. Conclusion: This is the largest retrospective patient study in the field. The potential benefits of ITB in selected patients with HSP were demonstrated. Keywords: baclofen, intrathecal baclofen, hereditary spastic paraplegia, gait analysis

Two brothers with butterfly-shaped macular dystrophy (BSMD) are reported. The anatomical and functional data were analyzed in 11 family members. Progressive photoreceptor dysfunction, supported by ERG abnormalities, was documented in both patients. The progression of the disease was observed over a period of seven years. The present observations emphasize that BSMD, at least in some cases, can be a chronic progressive disorder with secondary involvement of the photoreceptors.

A “designer drug” that touched off a mysterious epidemic of parkinsonism has provided insights into a basic pathologic process of the disease—destruction of dopaminergic neurons. These insights have also helped correlate clinical approaches with biomedical events, opening the way for development of more effective pharmacologic agents for a still relentlessly progressive disorder.